Recently, valve upgrade and/or endoscopic third ventriculostomy, which have the merit of no additional shunting, were introduced for the treatment of slit ventricle syndrome, because lumboperitoneal shunting entails various complications including development of Chiari malformation, shunt malfunction, and infection. However, the safety of valve upgrading is not confirmed, especially in a child with slit ventricle syndrome developed as a result of pseudotumor cerebri. A 5-year-old boy with pseudotumor cerebri presented with headache, intermittent vomiting, and sudden deterioration of visual acuity. His cerebrospinal pressure during lumbar puncture was 69 cm H(2)O and his magnetic resonance imaging revealed only small ventricles. He underwent a ventriculoperitoneal shunt resulting in dramatic improvement. Four months later, he returned with recurrent spontaneous valve malfunction with recurrent severe headache and visual deterioration. After shunt revision with a programmable valve, his intermittent valve malfunction was improved by upgrading the valve opening pressure. However, his visual acuity became progressively aggravated. He underwent a lumboperitoneal shunt with low-pressure valve, which resulted in the disappearance of intermittent headaches and a deterioration of visual acuity. We suggest that valve pressure upgrade in children with slit ventricle syndrome after ventriculoperitoneal shunt for pseudotumor cerebri may produce acute deterioration of vision that had already been compromised, even within the normal intracranial pressure range and with improvement of associated symptoms.

PURPOSE: The purpose of this work is to review the pathophysiology and treatment of severe headache disorders in patients having a shunt for hydrocephalus.

MATERIALS AND METHODS: The literature on the management of the slit ventricle syndrome is reviewed as well as an assessment of personal experiences over a 30-year period in the management of severe headache disorders in shunted patients. RESULTS: If the slit ventricle syndrome is defined as severe, life-modifying headaches in patients with shunts and normal or smaller than normal ventricles with ventricular shunts for the treatment of hydrocephalus, there are five different pathophysiologies that are involved in the process. These pathologies are defined by intracranial pressure measurement as severe intracranial hypotension analogous to spinal headaches, intermittent obstruction of the ventricular catheter, intracranial hypertension with small ventricles and a failed shunt (normal volume hydrocephalus), intracranial hypertension with a working shunt (cephalocranial hypertension), and shunt-related migraine. The treatment of these conditions and identifying patients with each condition are facilitated by attempting to remove the shunt.

CONCLUSIONS: Following the analysis of attempts to remove shunts, there are three possible outcomes. In about a quarter of patients, the shunt can be removed without having to be replaced. This is most common in patients treated in infancy for post-hemorrhagic hydrocephalus or patients shunted early after or before brain tumor surgery. Another half of patients have increased intracranial pressure and enlarged ventricles. In these patients, there is an 80% success rate for endoscopic third ventriculostomy. Finally, the most severe form of the slit ventricle syndrome involves intracranial hypertension without ventriculomegaly, which is managed optimally by shunt strategies that emphasize drainage of the cortical subarachnoid space such as lumbo-peritoneal shunts or shunts that include cisterna magna catheters.

Sood S, Barrett RJ, Powell T, Ham SD. The role of lumbar shunts in the management of slit ventricles: does the slit-ventricle syndrome exist?Journal of Neurosurgery. 2005 Aug;103(2 Suppl):119-23.

OBJECT: Slit-ventricle syndrome (SVS) is a confusing description of presentations in patients with chronic shunt-treated hydrocephalus. These patients are prone to acute deterioration with recurrent malfunction. The authors describe the clinical profile and management outcome of using lumboperitoneal (LP) shunts in this population of patients.

METHODS: Thirty-three patients with slit ventricles and recurrent malfunctions were converted to LP shunts (mean age 12 years). The initial ventricular shunt was placed at a mean age of 16.5 months. Ten patients had failed endoscopic third ventriculostomies prior to placement of their LP shunt. At a previous presentation, in 11 patients suspected to have SVS following revision of the shunt, intracranial pressure normalized after insertion of a contralateral shunt, suggesting that their ventricles were isolated. The rate of infection and malfunction was compared before and after conversion to an LP shunt. Twenty-seven patients were successfully converted to LP shunts. Four of the 11 patients with isolated ventricles required ventricular shunts in addition to the LP shunt. During a mean follow-up period of 16.7 months, the malfunction rate per patient decreased from 4.81 for ventriculoperitoneal shunts, prior to conversion to 1.48 after conversion to LP shunts, a statistically significant reduction (p < 0.000). No significant difference was found in the rate of shunt infections (7.1% for VP shunts and 9.6% for LP shunts, p = 0.44). No patient presented with acute symptoms following malfunction of an LP shunt or suffered from a Chiari I malformation.

CONCLUSIONS: Conversion to an LP shunt is a safe and effective procedure in patients prone to rapid decompensation and recurrent shunt malfunctions from small, slitlike ventricles. The term SVS is confusing. The condition is a manifestation of an unrecognized slitlike isolated ventricle and should be abandoned.

OBJECTIVE: Slit ventricle syndrome (SVS) has been described in hydrocephalus patients who continue to have shunt malfunction-like symptoms in the presence of a functioning shunt system and small ventricles on imaging studies. These symptoms usually present years after shunt placement or revision and can consist of headache, nausea and vomiting, lethargy and decreased cognitive skills. Treatments offered range from observation, medical therapy (migraine treatment) and shunt revision to subtemporal decompression or cranial vault expansion. We describe a subset of patients with SVS who were symptomatic with high intracranial pressure (ICP) as measured by sedated lumbar puncture and whose symptoms completely resolved after lumboperitoneal shunt (LPS) placement.

METHODS: Seven patients with a diagnosis of SVS underwent lumboperitoneal shunting. The age at shunting ranged from 3 to 18 years. Most had undergone recent ventriculoperitoneal shunt (VPS) revisions for presentation of shunt malfunction-like symptoms. Despite this, all remained symptomatic and underwent a sedated lumbar puncture to measure opening pressure (OP). All had high OP in spite of a functional VPS and underwent LPS placement.

RESULTS: All 7 patients had a prolonged period of overdrainage symptoms after lumboperitoneal shunting that resolved completely over several weeks. The initial etiology of hydrocephalus was reported to include trauma, aqueductal stenosis and intraventricular hemorrhage of prematurity. Two patients required revision of their LPS, after which their symptoms again resolved.

CONCLUSION: In a certain subset of patients with SVS who are symptomatic from increased ICP, placement of an LPS is an effective treatment option. It appears that this subgroup of patients previously treated with ventriculoperitoneal shunting behave in a fashion similar to pseudotumor cerebri patients and respond well to lumboperitoneal shunting.

To determine whether it is worth while to encourage patients who have high-level spina bifida to walk at an early age, we compared the cases of thirty-six patients who had participated in a walking program with those of thirty-six patients for whom a wheelchair had been prescribed early in life. The patients in the two groups were matched for age, sex, level of the lesion, and intelligence. Only twelve of the patients who had been able to walk at an early age were still able to do so effectively at the time of this study, when their ages ranged from twelve to twenty years, but still these patients fared somewhat better than the other patients did. The patients who walked early had fewer fractures and pressure sores, were more independent, and were better able to transfer than were the patients who had used a wheelchair from early in life. However, during childhood and early adolescence, the patients who had always used a wheelchair had spent fewer days in the hospital than did those who had participated in the walking program. There were no major differences between the two groups with regard to skills of daily living, function of the hands, and frequency and severity of obesity.